Pheochromocytoma is a rare tumor of the adrenal medulla composed of chromaffin cells and is part of the paraganglioma (PGL) family of tumors, being defined as an intra-adrenal PGL .[2][4][5] These neuroendocrine tumors can be sympathetic, where they release catecholamines into the bloodstream which cause the most common symptoms, including hypertension (high blood pressure), tachycardia (fast heart rate), sweating, and headaches.[6][7] Some PGLs may secrete little to no catecholamines, or only secrete paroxysmally (episodically), and other than secretions, PGLs can still become clinically relevant through other secretions or mass effect (most common with head and neck PGL).[8] PGLs of the head and neck are typically parasympathetic and their sympathetic counterparts are predominantly located in the abdomen and pelvis, particularly concentrated at the organ of Zuckerkandl at the bifurcation of the aorta.[9]
^ ab Gimenez-Roqueplo AP, Robledo M, Dahia PLM. Update on the genetics of paragangliomas. Endocr Relat Cancer. 2023 Mar 8;30(4):e220373. doi: 10.1530/ERC-22-0373. PMID: 36748842; PMCID: PMC10029328.
^Beard CM, Sheps SG, Kurland LT, Carney JA, Lie JT (December 1983). "Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979". Mayo Clinic Proceedings. 58 (12): 802–4. PMID6645626.
^Oyasu R, Yang XJ, Yoshida O, eds. (2008). "What is the difference between pheochromocytoma and paraganglioma? What are the familial syndromes that have pheochromocytoma as a component? What are the pathologic features of pheochromocytoma indicating malignancy?". Questions in Daily Urologic Practice. Tokyo: Springer Japan. pp. 280–284. doi:10.1007/978-4-431-72819-1_49. ISBN978-4-431-72819-1. {{cite book}}: |work= ignored (help)
^Williams MD. Paragangliomas of the Head and Neck: An Overview from Diagnosis to Genetics. Head Neck Pathol. 2017 Sep;11(3):278-287. doi: 10.1007/s12105-017-0803-4. Epub 2017 Mar 20. PMID: 28321772; PMCID: PMC5550402.
